Capillary diagnostics of the body. Capillaroscopy allows you to establish the correct diagnosis. It is important to identify improper blood circulation in capillaries and veins
Capillaroscopy is a method of intravital examination of capillaries and capillary circulation. Using capillaroscopy, it is possible to study the function of human skin capillaries under both physiological and various pathological conditions.
The discovery of capillaries is associated with the invention of the microscope. In 1681, Malpighi first discovered capillaries in the mesentery of a frog. Huther, with the help of a specially designed apparatus (1879), was the first to see capillaries on the mucous membrane of the human lip, and Lombard proposed a method for observing capillaries at the nail bed, using for this purpose the enlightenment of the epidermis with glycerin or some liquid oil (1911). The basis for modern capillaroscopy and its application was the work of Müller (1916-1922), who also used the skin lightening technique.
The capillaries of the nail bed, due to their horizontal location, are accessible to study along their entire length, in contrast to areas of the skin in other places, where for the most part it is possible to see only the tops of the capillary loops.
The convenience of observing capillaries at the edge of the nail fold is also facilitated by their more superficial location in this area; Finally, the finger is very convenient for microscopy.
Capillaroscopy is carried out at low magnification (20-70-100 times) using a microscope or a special device - a capillaroscope, which, in addition to the optical system, has a constant light source and a device for fixing a finger. Some capillaroscope systems are equipped with a photo attachment for capillarography. A good capillaroscope is suitable for examining skin capillaries on any part of the human body, as well as the mucous membrane of the lip and tongue. The grid inside one of the eyepieces makes it possible to make measurements during capillaroscopy with an accuracy of 0.05 mm.
In strong lighting, during long-term studies with artificial light, one should keep in mind the possibility of temperature effects on the skin, which also affects the condition of the capillaries. To eliminate the temperature factor, it is recommended to pass light through a special cooling device.
Carrying out capillaroscopy
Capillaroscopy is performed as follows. 2-3 minutes before the start of the study, one or two drops of glycerin, cedar or vaseline oil are applied to the surface of the skin of the nail fold; This achieves enlightenment (homogenization) of the epidermis. Then the finger (usually the fourth finger of the left hand as the least injured) is placed on a special stand (for better fixation) so that the capillaroscope lens is above the cleared area, and is illuminated with strong lateral light. To avoid physical stress affecting blood circulation, the hand is placed in a horizontal position on a bag of sand. By changing the position of the capillaroscope tube, they find the capillaries, examine them, and then sketch them on paper.
In order to make capillaroscopy more objective, some capillaroscope systems have a projection-drawing device, with the help of which it is possible to copy visible capillaries and their morphological features or a photographic attachment for photographing visible capillaries.
The appearance of capillaries under a microscope during capillaroscopy depends on their position relative to the surface of the skin. If they run parallel to the surface, they have the appearance of elongated light red loops in the shape of a wire ladies' head pin; if they are located at an angle, then depending on its size - in the form of a curved comma or just a red dot. A normal capillary loop consists of a narrow ascending arterial limb and a wider, sometimes with slight waviness, descending venous one. The place where they connect is called the apex or apex of the capillary loop.
The color of the capillaries is intensely red. They are located in rows, in the form of a palisade, on several floors. During capillaroscopy, you should also pay attention to the background color of the entire microscopic field, the presence of a pronounced subpapillary layer, pathological forms of capillaries and the nature of blood flow in them. The background of the microscopic field under normal conditions has a pale pink color, the capillary loops are usually of a uniform shape, in the form of a lady's hairpin, red. The number of capillaries over 2 mm is 16-20 pieces.
On average, in the field of view (at 80x magnification) there are 40-60 capillaries, and in the first row, where the capillaries are most correct both in height and in the intervals between them, there are 14-17 of them. The vessels of the subpapillary layer are usually not pronounced. Only in some cases, with thin skin, are the afferent arterioles, branches of the first subpapillary venous network, visible. The blood flow in the capillaries is uniform and fast, so that it is almost invisible to the observer's eye. Capillaries in these cases appear as uniformly red threads. With careful observation during capillaroscopy, it is possible to detect the movement of blood flow, and it is better visible in the area of the apex of the capillary loop; its direction is always from the arterial to the venous knee, which helps to distinguish the latter. In some cases, slow blood flow may occur.
In pathological cases, the capillaroscopy picture changes. The background of the microscopic field becomes pale or, conversely, darker with various shades of cyanosis; sometimes there is a cloudy background. The capillaries take the shape of a figure eight, become tortuous or multiply tortuous with aneurysmal extensions. The capillaries are connected to each other using anastomoses. In other cases, the capillaries, on the contrary, are very small due to the reduction and narrowing of the arterial and venous branches. In these cases, they take the form of fragments, commas, and periods. The number of capillaries can change either increasing or sharply decreasing (up to 3-7). Their arrangement becomes unsystematic, dense or, conversely, sparse, even with completely avascular fields. In cases of stagnation, the vessels of the subpapillary layer are often clearly visible. The movement of blood in the capillaries slows down, sometimes to a complete stop, or becomes intermittent. In this case, the passage of individual red blood cells or their clusters is clearly visible. It seems that the blood in them is so viscous and thick that it hardly passes from one knee to another. In these cases, the capillaries do not appear homogeneous, but granular.
Capillaroscopic examination must be carried out in a certain sequence. First, the entire nail fold is examined. At the same time, attention is paid to the background of the microscopic field, the shape of the capillaries and the nature of the blood flow in them. After this, any of the fields of vision are examined in detail. The number of capillaries is counted, the presence of anastomoses and aneurysmal dilatations is recorded, and the nature of the blood flow is studied. All this data is sketched and described in protocols or photographed.
Capillaroscopy results
A comparison of capillaroscopic data for various diseases of internal organs, the nervous system, blood diseases, and metabolism showed that changes in the shape of capillaries, the number of functioning loops, the nature of the blood flow in them and the background of the microscopic field do not represent anything strictly typical for each of these diseases. Capillaroscopic pictures can be the same for completely different diseases, while at the same time different stages of the same disease can manifest themselves with sharply different capillaroscopy data. Thus, changes in capillaries observed in cardiovascular failure occur in patients with diabetes mellitus; The capillaroscopic picture in case of myocardial infarction is identical, but in case of vasoneurosis it differs in the variety of capillary shapes and their functional instability. This circumstance significantly reduces the diagnostic value of capillaroscopy, although it allows one to expand the understanding of the depth of existing changes in the body and to a certain extent helps in assessing the state of blood circulation, especially during dynamic observation and with clinical data and other research methods.
The capillaroscopy method is of great value for diseases characterized by functional or organic changes in peripheral blood vessels: obliterating endarteritis, atherosclerotic vascular occlusions, congenital and traumatic aneurysms.
A certain constancy of the capillaroscopic picture, as well as the presence of inadequate or perverted vascular reactions, make it possible to speak more definitely about the nature of the disease, the severity of the process and the prognosis. Thus, the following picture is characteristic of obliterating: a cloudy background, sometimes with a cyanotic tint, a sharply reduced number of capillaries, tortuous capillary loops, the arterial limb is shortened and thinned, the venous limb is often dilated; There are deformed hook-shaped capillaries. The blood flow is slow, granular, and sometimes difficult to detect. Some have used capillaroscopy to determine the stage of obliterating endarteritis. Its initial forms are characterized by deformation of the capillaries, their sharp narrowing and an increase in the number of capillaries; for the stage of trophic disorders and necrosis - a decrease in the number of capillaries, the presence of avascular fields. In clinically different forms of obliterating endarteritis - atonic, spastic and spastic-atonic - changes in the capillaries are also quite clearly differentiated.
More clearly, capillaroscopic changes are detected after various functional tests: with nitroglycerin, hot baths, physical activity, and with tests according to A. I. Nesterov and Lange. To establish indications for lumbar sympathectomy, it is recommended to perform a novocaine blockade of the second sympathetic lumbar node. If after this there is an improvement in capillary circulation, the operation is considered indicated.
Capillaroscopy during treatment and after its completion allows a number of patients with obliterating endarteritis to judge the effectiveness and results of the therapy.
Thus, capillaroscopy as a diagnostic method has relatively little independent significance and in clinical practice only complements and deepens the data obtained using other methods for studying peripheral vessels. In case of obliterating endarteritis, due to the parallelism between the nature of the pathological process and changes in the capillaroscopic picture, capillaroscopy data make it possible to a certain extent to judge the course of the disease, its form and stage, as well as the effectiveness of treatment and prognosis.
Errors during capillaroscopy may depend on insufficient clearing of the capillaroscopic field, as well as when examining only one field of view.
In diseases characterized by intermittent spastic reactions, an incorrect conclusion may be obtained as a result of short-term observation. When performing capillaroscopy, the temperature factor and previous physical activity should be taken into account. When examining toes, changes in the capillaroscopy pattern may depend on the wearing of shoes. During manicure or pedicure, the finger is not suitable for capillaroscopy.
The article was prepared and edited by: surgeonScleroderma is a chronic connective tissue disease that affects the skin and some internal organs of the human body. The basis of this disease is a violation of the properties of the connective tissue framework of organs, which leads to the occurrence of sclerotic changes, that is, to the appearance of coarse non-functional fibrous fibers ( scar tissue).
Scleroderma is an autoimmune rheumatological disease. The term “autoimmune” means that the basis of this disease is a disruption of the immune system, which for some reason begins to “attack” the cells and tissues of its own body. The consequence of this is an inflammatory reaction, which leads to thinning and hardening of the skin, blood vessels, and a number of internal organs ( such as the esophagus, lungs, kidneys, stomach, intestines, heart).
Despite the different localization of lesions, it is impossible to clearly distinguish the forms of scleroderma. Moreover, many prominent researchers believe that both forms of the disease are a consequence of the same pathological process.
Scleroderma is a pathological condition that significantly complicates the life of patients. This is due, first of all, to the limitation of physical activity, as well as to the pain that can occur in some situations. Due to digestive problems, such patients often need a special diet, they are forced to eat often and in small portions. Due to degenerative-sclerotic changes in the skin, patients have to constantly monitor the level of hydration and also be extremely careful when playing sports or any other physical activity.
In addition, many patients with scleroderma experience psychological discomfort associated with thoughts about the disease, which is chronic and currently incurable. Since this disease can cause significant changes in appearance, self-esteem and self-image suffer, which leads to various social and personal conflicts.
Psychological support for patients with scleroderma, which should be provided by family members, friends and loved ones, is extremely important and helps maintain an adequate quality of life.
Interesting Facts
- scleroderma is a Greek term that comes from the words skleros ( solid) and derma ( leather);
- the term "scleroderma" was introduced into medical practice in 1847;
- the first description of the clinical picture of scleroderma was made back in the 17th century;
- Among autoimmune diseases, scleroderma is one of the most common ailments;
- more than 75% of patients with scleroderma are women;
- The disease can affect adults and children, but it is mainly registered in the age group from 30 to 50 years.
Causes of scleroderma
Scleroderma is an acquired chronic connective tissue disease, the exact cause of which still remains unclear. However, thanks to the development of medicine, molecular biology and laboratory diagnostics, it has become possible to identify and study the main pathological mechanisms involved in the development of the disease. Today, there are several theories that describe factors that can cause scleroderma, as well as the mechanism of their action. It is believed that the following factors may be involved in the development of scleroderma:
- genetic factor;
- inflammatory factor;
- autoimmune process;
- infectious factor;
- environmental factors;
- a number of medications.
Genetic factor
To date, the question of how large the role of genetic factors in the development of scleroderma still remains open. It is undeniable that there is a certain genetic predisposition to the disease, which is confirmed by a higher incidence among first-generation relatives, but the mechanisms that can trigger the work of pathological genes are not entirely clear.Some studies have found that the occurrence of scleroderma is most likely associated with a defect in the HLA-9QA1 gene. This gene is responsible for the synthesis of proteins that play a key role in the functioning of the immune system. These proteins, called Human Leukocyte Antigen ( human leukocyte antigen - HLA), are presented on the surface of a number of cells and serve to ensure that the immune system can distinguish its own biological material from bacteria and viruses and does not attack its cells. Changes in the structure of this gene and, accordingly, in the structure of cell surface proteins lead to various autoimmune reactions and, according to several studies, are quite often detected in patients with scleroderma. There are several other genes involved in the regulation of immune processes, the defeat of which can cause the development of this disease. Unfortunately, studies conducted to date reveal rather contradictory data on the involvement of certain genes in the development of the disease.
The main point in the development of scleroderma is damage to the connective tissue, which can occur for many reasons, including due to genetic defects. Damage to genes responsible for the production of a factor that regulates the growth of connective tissue, as well as a factor that controls the formation of fibroblasts ( cells that synthesize connective tissue), is one of the potential causes of scleroderma.
Changes in the genetic apparatus can be either congenital or acquired. It should be noted that scleroderma, as such, is not inherited, however, people who are closely related to patients have a much higher chance of developing the disease.
Changes in the structure of genes are called mutations. They arise spontaneously as a result of random rearrangements of nucleotides ( structural acids encoding genetic information) during the division of ordinary ( somatic) and germ cells. In order for the protein encoded by the gene to acquire a pathological structure, it is enough that just one nucleotide is replaced by another. However, it should be understood that the human genome has repair mechanisms that successfully eliminate most of the consequences of incorrect replication ( reproduction) chromosomes.
Genome mutations can occur under the influence of the following factors:
- ionizing radiation;
- ultraviolet radiation;
- high and low temperatures;
- nitrates;
- pesticides;
- some medications ( cytostatics);
- nutritional supplements;
- solvents;
- viruses;
- antigens of some bacteria.
Inflammatory factor
The inflammatory reaction is a factor that, under certain circumstances, can cause the development of scleroderma. This is due to the fact that during the inflammatory reaction, the production of various biologically active substances increases, which can affect tissues and cause functional and structural changes in cells and blood vessels. Due to this, vascular permeability increases, conditions are created under which immunocompetent cells are “attracted” to the inflammatory focus. All this creates the prerequisites for the activation of fibroblasts ( cells that synthesize connective tissue fibers). As a result, a chronic inflammatory reaction occurs, which, against the background of genetic abnormalities or the influence of a number of other factors, triggers systemic scleroderma.Autoimmune process
The autoimmune process is a consequence of a disruption in the normal functioning of the immune system and is a pathological immune response directed against the body's own tissues.The occurrence of an autoimmune process may be associated with the following factors:
- Genetic abnormalities. As mentioned above, with genetic pathologies, the synthesis of normal surface protein may be disrupted, which leads to a situation in which immune cells do not recognize their own tissues and attack them.
- Pathogenic agents. Some pathogenic agents have antigens that are similar in structure to human proteins. This allows them to protect themselves from the immune system, but when a response develops, this creates the preconditions for a parallel attack by immune cells and antibodies on bacteria and their own tissues.
- Other reasons. Autoimmune processes are extremely complex and can occur for many reasons.
Infectious factor
For a long time, infectious agents were considered as a factor capable of initiating scleroderma. However, to date it has not been possible to identify a specific infection that could act as a provoking factor.However, it is believed that some infectious agents are capable of triggering autoimmune reactions by affecting the immune system and thereby provoking the development of systemic scleroderma. Some patients had serum antibodies to protein fragments of human cytomegalovirus, which under certain conditions can interact with normal body tissues. This is due to the fact that the proteins of the virus have the ability to mimic, that is, they form structures similar in structure to the proteins of the human carrier. Thanks to this mechanism, during the development of an immune response, immunocompetent cells attack not only the antigens of the infectious agent, but also their own tissues.
Thus, antibodies to cytomegaly virus fragments have the following effects on the body:
- Activation of fibroblasts. Fibroblasts are body cells that synthesize connective tissue fibers. They are activated by anticytomegalovirus antibodies, as well as under conditions of hypoxia, tissue damage, and inflammatory reactions. At the same time, due to excessive synthesis of collagen, which forms a rigid frame, the tissues lose their original elasticity.
- Proliferation of vascular smooth muscle. Under the influence of antibodies, the proliferation of vascular smooth muscle cells is stimulated, which leads to a narrowing of their lumen and, accordingly, to hypoxia. This is fraught with progressive cell damage, as well as a slight increase in connective tissue synthesis.
- Formation of a new inner lining of blood vessels. Under the influence of human cytomegalovirus, vascular intima ( membrane lining the inner surface of blood vessels) thickens, which leads to a narrowing of its lumen.
Environmental factors
Medical research has found that there are geographic areas in which the incidence of scleroderma is slightly higher than in other areas. A more detailed study of this phenomenon suggested that there are certain environmental factors that in one way or another are capable of influencing the body and initiating systemic scleroderma.Exposure to the following chemicals can trigger scleroderma:
- silicon;
- heavy metals;
- mercury;
- organic solvents;
- vinyl chloride;
- benzene;
- toluene;
- trichlorethylene;
- epoxy resin;
- urea-formaldehyde resin;
- paraffin and silicone ( used for cosmetic breast augmentation).
- L-tryptophan;
- mazindol
- fenfluramine;
- amfepramone.
Exposure to environmental factors can stimulate both local and systemic responses. A pathological process initiated by any factor continues even after its influence has ceased.
It should be noted that exposure to certain chemicals and environmental factors can provoke sclerotic changes in the skin or internal organs, which, however, are not elements of scleroderma. For example, under the influence of alcohol, a skin pathology occurs, which is externally similar to scleroderma, but is not it.
It should be understood that these factors can cause the development of scleroderma only if there is an appropriate genetic background.
A number of medications
There are quite a large number of medications that, under certain conditions, can cause sclerosis of the skin and internal organs.The following drugs can act as a local provoking factor:
- phytomenadione;
- pentazocine;
- heparin.
The following medications can cause systemic scleroderma:
- bleomycin;
- L-tryptophan;
- carbidopa;
- penicillamine;
- sodium valproate;
- cocaine;
- amphetamine;
- diltiazem.
Symptoms of scleroderma
In medical practice there are limited ( localized) scleroderma, in which lesions are limited to the skin, muscles, joints and bones, and the systemic form, in which changes occur in the blood vessels, lungs, kidneys, heart and other internal organs. Scleroderma manifests itself with various clinical symptoms, which depend on the location of the process and, accordingly, on the affected organs. An important factor influencing the manifestations of pathology is the stage of its development and the degree of sclerotic changes.
In the initial stages of scleroderma, the following nonspecific symptoms may occur:
- malaise;
- muscle pain.
Further symptoms of scleroderma depend on the localization and distribution of sclerotic foci.
In clinical practice, it is customary to distinguish the following forms of the disease:
- Limited scleroderma. With limited scleroderma, separate lesions appear in the thickness of the skin, sometimes in muscles and bones. Damage to peripheral blood vessels develops. Depending on the shape and type of lesions, plaque, linear and patchy scleroderma are distinguished.
- Systemic scleroderma. With systemic scleroderma, developing lesions are not limited to skin lesions and spread to internal organs, causing disruption of their function.
Skin lesions
In the evolution of limited skin lesions, three successive stages are distinguished:
- Edema. The inflammatory reaction that occurs initially is accompanied by the release of biologically active substances that cause vasodilation, which leads to edema. As a result, the fingers, hands and feet increase in volume, and some swelling and pastiness are observed ( when pressed, a trace remains for some time), skin folds are smoothed out. In some cases, itching develops. Skin color can be bright red or red with a bluish tint. This stage lasts for several weeks.
- Seal. At the stage of compaction or sclerosis, the normal structure of the connective tissue of the skin is replaced by a pathological one. Because of this, waxy-yellow foci of sclerosis appear, surrounded by a bluish-purple growth zone. In the area of these lesions, the skin is hard, cold, inseparable from the underlying tissues.
- Atrophy. Atrophy is the final stage of the process. At this phase of the pathology, the skin becomes thinner, loses its color, and resembles parchment paper. Due to damage to the sweat and fat glands, the skin is dry and easily cracks under the influence of any factors. When subcutaneous fatty tissue and muscles are affected, the skin may adhere directly to the underlying bone structures.
Depending on the distribution and type of sclerotic lesions on the surface of the skin, several forms of the disease are distinguished, among which the most common is plaque scleroderma, so named because of the shape of the primary lesion, which forms plaques on the surface of the skin.
Forms of scleroderma depending on the type of sclerotic lesions
| Form of scleroderma | Characteristic | Photo |
| Plaque scleroderma | Plaque scleroderma is the most common form of the disease. Initially, edematous lesions up to 1–5 cm in size, round in shape, pinkish in color appear on the skin, which gradually increase and spread ( at the same time, progressive compaction is observed in the center). At the stage of atrophy development, the skin becomes thin, it becomes recessed and becomes pale. In some cases, areas of calcium deposits form in the lesions. |  |
| Linear scleroderma | Linear scleroderma is more common among children. It is characterized by one lesion located on the scalp, which gradually spreads to the forehead and nose. Due to its linear distribution, this lesion resembles a scar from a saber strike. When localized in other parts of the body ( rarely) sclerotic changes spread along the nerve trunks. |  |
| White spot disease | White spot disease causes small, up to one centimeter in diameter, round and shiny white lesions. These spots are characterized by a dense structure and a raised surface. A reddish growth zone is visible at the border with healthy tissue. Most often, lesions are located on the neck, less often on the torso ( shoulders, chest), as well as on the mucous membrane of the oral cavity and genital organs. |  |
| Bullous scleroderma | Bullous scleroderma is characterized by the formation of blisters on the surface of the skin. It is the result of damage to the lymphatic vessels in combination with the release of biologically active substances from eosinophils ( one type of leukocyte). With this form of the disease, other types of sclerotic foci are usually detected. |  |
| Limited scleroderma with unilateral progressive facial atrophy | This form of scleroderma can occur either independently or in combination with other forms of the disease. Usually observed in people under the age of 18 – 22 years. Sclerotic changes begin in the area of the eye, zygomatic arch and lower jaw. In this case, the skin atrophies, bypassing the stage of edema and sclerosis. Due to the involvement of the underlying tissues, severe chronic pain occurs. The process is accompanied by loss of hair, eyelashes, and eyebrows. Unlike other forms of scleroderma, the activity of the sweat and fat glands in this type of disease does not decrease, but, on the contrary, increases. Due to muscle and bone atrophy, as well as nerve damage, the face loses symmetry, and the affected half decreases in size. |  |
Spider veins and areas of calcification form in areas of the affected skin. Spider veins ( telangiectasia) most often occur on the face, mucous membranes of the mouth, eyes, genitals, chest, and arms. Subcutaneous calcinosis occurs when calcium salts are deposited in the thickness of the skin or subcutaneous fat. This process usually occurs in areas subject to frequent trauma ( anterior surface of the forearms, fingertips). Calcium deposits can erode the skin over time and form ulcers.
Vascular damage
The action of pathogenic mechanisms in scleroderma is not limited only to the skin, but also affects blood vessels. In almost all patients with this disease, vascular damage manifests itself in the form of Raynaud's syndrome - spasm of peripheral arteries and arterioles under the influence of low temperatures or stress. Usually this phenomenon affects the fingers, but in rare cases it can also appear on the feet and even on the tongue. Classically, Raynaud's phenomenon is described as a three-phase change in the color of the skin of the fingers. With Raynaud's phenomenon, the following phases of skin color change are detected:
- Paleness of fingers. Under the influence of cold, excessive spasm of arteries and arterioles occurs ( vessels that carry oxygen-rich blood away from the heart), which leads to a decrease in blood supply and, accordingly, to tissue ischemia and skin pallor. There is usually a clear boundary between the pale fingers and the normal-colored hand.
- Blue fingers. The developed spasm of blood vessels and insufficient supply of nutrients and oxygen lead to the fact that hemoglobin begins to accumulate in the fingers, which gives up oxygen and combines with a carbon dioxide molecule, which gives the skin a bluish appearance ( cyanotic) shade.
- Redness of fingers. After eliminating vascular spasm, redness occurs, which is associated with reactive plethora ( blood rushes into exhausted and incapable of adequate contraction peripheral vessels).
In addition to changes in skin color, Raynaud's phenomenon may cause the following symptoms:
- numbness of fingers;
- tingling in fingers;
- local discomfort during an attack.
In some cases, with a long history of the disease, Raynaud's phenomenon may be accompanied by the following symptoms:
- poorly healing ulcers on the fingers;
- dry gangrene of fingers;
- spontaneous amputation of affected fingers ( death of fingers).
Damage to internal organs
With systemic scleroderma, the following organs and organ systems can be affected:- gastrointestinal tract;
- liver;
- lungs;
- heart;
- kidneys;
- bones and muscles;
- genitourinary system.
Damage to the gastrointestinal tract ( Gastrointestinal tract) is one of the most common manifestations of systemic scleroderma among patients who do not have Raynaud's phenomenon. The entire digestive system may be affected, but more often sclerosis occurs in the oropharynx, esophagus, stomach, large and small intestines, and rectum. The salivary glands may be damaged, leading to difficulty swallowing.
The basis of gastrointestinal lesions is smooth muscle atrophy ( due to damage to blood vessels and nerve endings) and fibrosis ( replacement of normal connective tissue). All this leads to disruption of peristalsis and transit of the contents of the digestive system. Sclerosis of the intestinal mucosa leads to impaired absorption of nutrients, which can lead to weight loss and insufficient intake of essential vitamins and minerals.
Damage to the gastrointestinal tract is accompanied by the following symptoms:
- reduction in the volume of the oral cavity;
- dry mouth;
- swallowing disorder;
- heartburn;
- involuntary regurgitation of gastric contents;
- narrowing of the esophagus ( manifested by an early feeling of fullness, heaviness in the sternum, regurgitation of undigested food, weight loss);
- early saturation;
- hidden intestinal bleeding;
- vomiting blood;
Liver
Scleroderma rarely directly affects liver tissue, but in some cases it can act as a factor contributing to the development of primary biliary cirrhosis. Moreover, due to an increase in bilirubin levels ( a breakdown product of hemoglobin, which is normally processed by the liver and excreted along with feces) jaundice occurs. When the bile ducts become sclerotic, colorless feces, jaundice, and itching may occur.
Lungs
Lung damage is the most common cause of death among patients with scleroderma.
Damage to the lungs with scleroderma may have the following character:
- Interstitial lung disease. The interstitium is the framework of an organ, which consists of connective tissue, blood and lymphatic vessels, as well as tissue surrounding the vessels and nerves. With interstitial lung disease, sclerosis of the capillaries and walls of the alveoli occurs, which significantly complicates gas exchange processes in the lungs. As a result, shortness of breath develops, lung mobility decreases, and tidal volume decreases.
- Pulmonary hypertension. Increased pressure in the pulmonary artery system is called pulmonary hypertension. Hypertension develops due to a decrease in the internal lumen of blood vessels, as well as due to a decrease in the elasticity of the vascular wall, which leads to an increase in resistance to blood flow. As a result, the load on the right side of the heart increases, and progressive hypertrophy of the right ventricle occurs. Due to increased pressure in the right side of the heart, venous return decreases, which leads to stagnation of blood in the liver and some other organs of the systemic circulation. As a result, shortness of breath, chest pain, pain in the right hypochondrium, and swelling of the jugular veins are observed.
Heart damage in scleroderma is quite common, but clinical manifestations are absent in many cases. It is believed that clinically pronounced damage to the heart muscle and pericardial structures in scleroderma indicates an unfavorable course of the disease.
Heart damage in scleroderma can manifest itself with the following clinical signs:
- chest pain;
- shortness of breath during exercise;
- feeling of heartbeat;
- dizziness;
- swelling of the jugular veins;
- heart rhythm disorder ( arrhythmias).
Kidneys
With scleroderma, the renal vessels are affected, which leads to impaired renal function up to the development of acute renal failure. As a result, the amount of blood filtered through the kidneys decreases, and breakdown products begin to accumulate in the body, causing various adverse effects.
The disease usually manifests itself as arterial hypertension, which occurs in response to decreased blood flow in the renal artery system, and which is aimed at maintaining and restoring proper blood supply. In rare cases, the pressure increases slightly, but in patients whose blood pressure levels were normal before the onset of the pathology, it increases quite significantly. This is accompanied by shortness of breath, headaches, the appearance of “spots” or dots before the eyes, fainting, pulmonary edema, and edema of the lower extremities.
It should be noted that an increase in blood pressure against the background of kidney damage in scleroderma is a completely favorable sign, as it indicates normal function of the heart muscle.
Against the background of renal failure, peripheral edema and intoxication with decay products develop ( headaches, impaired consciousness). In some cases, effusion may form in the serous bursae - in the abdominal cavity ( dropsy, accompanied by an increase in the volume of the abdomen and compression of the abdominal organs), in the pleural cavity ( accompanied by respiratory failure) and in the pericardium ( accompanied by heart failure).
Due to impaired renal function, anemia develops, which is accompanied by pallor of the skin and decreased resistance to physical activity. There is also a platelet deficiency, which is accompanied by bleeding of the gums and mucous membranes of the gastrointestinal tract.
Bones and muscles
Many patients note that their earliest symptoms of scleroderma were associated with damage to the musculoskeletal system. In most cases, these symptoms are associated with pain during exercise and at rest. However, in some cases, the occurrence of pain in the joints and muscles may be associated with a true inflammatory reaction. The joints most often affected are the joints of the fingers and hands, as well as the wrist and elbow joints. Due to thinning of the skin and muscle atrophy, it is not always possible to adequately assess the degree of joint enlargement. Progressive damage to joints and muscles can cause the formation of flexion contractures ( significant limitation of limb mobility in the joint).
Genitourinary system
Damage to the genitourinary system with scleroderma is accompanied by the following symptoms:
- erectile disfunction;
- sclerosis of the bladder with a decrease in its volume ( as a result – frequent urge to urinate);
- pain during intercourse ( due to disruption of the vaginal glands and vaginal dryness);
- ulceration of the vaginal mucosa;
- decreased sexual desire;
CREST syndrome
CREST syndrome is an acronym for English terms that represent a list of the most common manifestations of systemic scleroderma.CREST syndrome includes the following pathologies:
- C – Calcinosis – Calcinosis. Calcinosis is dense subcutaneous formations that are deposits of calcium salts. In some cases, they form ulcers into which bacterial agents enter that can cause infection of soft tissues and bones ( osteomyelitis).
- R - Raynauld's phenomen - Raynaud's phenomenon. Raynaud's phenomenon, as previously described, occurs as a result of spasm of peripheral vessels and is manifested by impaired blood circulation in the fingers and toes. Characterized by a change in the color of the fingers when exposed to low temperatures.
- E – Esophageal dysmotility – impaired mobility of the esophagus. Due to damage to the smooth muscles of the esophagus, the swallowing process is disrupted, patients experience involuntary regurgitation and heartburn.
- S – Sclerodactyly – sclerodactyly. Sclerodactyly is a pathological condition in which the skin of the fingers is thickened, the subcutaneous tissue is atrophied, and the terminal phalanges are enlarged. Against the background of all these changes, the mobility of the fingers is impaired, and squeezing the hand becomes much more difficult.
- T – Telangiecasia – spider veins. Due to damage to the capillaries and other small blood vessels of the skin, small red-blue dots, similar in shape to stars, appear on the face, upper half of the body and on the mucous membranes of the mouth, eyes and genitals.
Diagnosis of scleroderma
Diagnosis of scleroderma is a dynamic process that requires constant monitoring and monitoring of the patient's condition. This is due to the fact that this disease is chronic and constantly progresses. This leads to the fact that after a certain period of time, patients develop new lesions in the internal organs and their general condition is disrupted. Diagnosis of scleroderma is based on periodic monitoring of patients with identification of the localization of sclerotic foci and determination of the degree of damage to internal organs, as well as a number of additional laboratory tests and procedures. The greatest value in this process is the clinical manifestations of the disease.
Clinical manifestations
In 1980, the American Rheumatological Association proposed criteria based on clinical symptoms that, when identified, can be diagnosed as scleroderma. These criteria are roughly divided into major and minor, depending on how often they occur and how specific they are. The presence of only a few combinations of symptoms indicating scleroderma greatly facilitates the process of differential diagnosis.Large criteria:
- Skin damage. Sclerosis of the skin, which spreads from the base to the top of the fingers or toes.
- Sclerodactyly. Thickening of the skin of the fingers and hands with limited mobility and expansion of the nail phalanx.
- Scars on the fingertips. The presence of fresh ulcers or scars on the palmar surface of the nail phalanges.
- Basal pulmonary fibrosis ( pneumofibrosis). With basal pneumofibrosis, connective tissue grows in the basal ( lower) parts of the lungs. With scleroderma, the process is symmetrical. Manifested by shortness of breath and decreased vital volume of the lungs. Identified during radiological examination ( plain chest x-ray).
To make a diagnosis of scleroderma, one major and at least two minor criteria must be present in one patient.
The remaining symptoms that are not included in these diagnostic criteria are also extremely important in the diagnosis process, as they allow us to determine the type of illness, its stage, and also indicate the organs involved in the pathological process.
Lab tests
Laboratory tests allow you to assess the condition of the whole organism and identify the main metabolic, structural and functional disorders.For scleroderma, the following laboratory tests are performed:
- General blood analysis. A complete blood count can detect a decrease in the number of red blood cells ( anemia), which may occur against the background of hidden bleeding or impaired renal function. A decrease in platelet count may also be detected. Erythrocyte sedimentation rate ( ESR) may be increased ( nonspecific sign of an inflammatory process), however quite often it is within the normal range. An increase in ESR is a sign of an unfavorable course of the disease.
- Blood chemistry. A biochemical blood test allows you to detect salts, enzymes, proteins and pigments contained in blood serum. In scleroderma, levels of muscle enzymes may be increased ( creatine phosphokinase, aldolase) due to the inflammatory process in the muscles. Elevated levels of creatinine, urea, and other nitrogen breakdown products indicate renal dysfunction. An increase in bilirubin levels indicates damage to the liver or bile ducts. Increased levels of alanine aminotransferase and aspartate aminotransferase enzymes ( ALT and AST), which usually indicate liver damage, are not informative in scleroderma, since these enzymes can be released from damaged skeletal muscles and the heart.
- General urine analysis. With kidney damage, a general urine test reveals increased levels of proteins and hydroxyproline, as well as a sediment consisting of red blood cells.
- Determination of CXCL4 level. CXCL4 is a biologically active substance that is secreted by plasma cells and interferes with the process of formation of new blood vessels. In systemic scleroderma, the level of this substance may be elevated, indicating pulmonary fibrosis and progressive pulmonary hypertension.
- Determination of NT-proBNP level. Activation of brain natriuretic peptide occurs due to the cleavage of the NT-proBNP fragment, the level of which in the blood can be determined using a number of laboratory tests. Brain natriuretic peptide is a hormone that is synthesized by the heart muscle in response to excessive exercise. Thus, NT-proBNP concentration correlates with the severity of pulmonary hypertension.
- C-reactive protein. C-reactive protein is a marker of the acute stage of the inflammatory response. In scleroderma, its level indicates disease activity.
Autoantibodies
Autoantibodies are immunoglobulins that can interact with self-antigens, that is, with the body's own tissues. The formation of these antibodies underlies many autoimmune and rheumatic diseases. Elevated levels of autoantibodies can be detected long before the clinical picture develops.The level and type of autoantibodies are important for making the initial diagnosis and for identifying associated pathologies, but they do not allow monitoring disease activity.
In scleroderma, the following types of autoantibodies can be detected:
- Antinuclear antibodies. Antinuclear antibodies ( A.N.A.) are detected in 90% of patients with scleroderma. They are immunoglobulins that are capable of attacking the contents of cell nuclei. There are several types of these antibodies, among which the most common are anticentromere antibodies and antibodies to topoisomerase I.
- Antibodies to topoisomerase I. Antibodies to topoisomerase I ( anti-Scl-70) are detected in 30% of patients with diffuse scleroderma. Patients with high levels of these antibodies are at increased risk of developing pulmonary fibrosis and interstitial lung disease.
- Anticentromere antibodies. Anticentromere antibodies are detected in almost half of patients with a limited form of scleroderma. Anticentromere antibodies are often associated with severe vascular damage and severe Raynaud's phenomenon.
- Anti-RNA polymerase I and III. Antibodies to RNA polymerase I and III are detected in a fifth of patients with diffuse scleroderma. These antibodies correlate with rapidly progressive skin lesions and a high risk of developing renal failure. Often found in people with several types of autoimmune diseases.
- Antiribonucleoprotein antibodies. Antibodies to ribonucleoproteins ( Anti-RNP) are detected in patients with several autoimmune diseases. Indicate skeletal muscle damage and interstitial lung disease.
Radiological research methods
Radiological research methods, that is, methods based on the use of x-rays, make it possible to detect changes in the structure of muscles, bones and a number of internal organs.In clinical practice, simple radiography and computed tomography are used. CT scan ( CT) is a more sensitive and modern diagnostic method, however, it involves a slightly higher radiation dose compared to simple radiography, and its use is not always justified.
Plain radiography is used to diagnose the following conditions:
- identifying subcutaneous foci of calcification ( which appear as intense foci of darkening);
- monitoring the condition of the nail phalanges of the fingers;
- exclusion of osteomyelitis in ulcers on the fingers;
- exclusion of acute intestinal obstruction due to impaired intestinal motility;
- detection of symmetrical basal pneumofibrosis ( increase in the intensity of the pulmonary pattern in the basal regions).
- fibrosis of the basal parts of the lungs;
- interstitial lung disease.
Electrocardiography
Electrocardiography ( ECG) is a method of routine examination of patients, which allows identifying structural and functional changes in the heart muscle. Using an ECG, you can identify signs of pulmonary hypertension, arrhythmia, and signs of heart failure.Ultrasonography
Ultrasound examination allows us to evaluate the structure and function of some internal organs without any risks to the patient.Ultrasound examination allows to identify pathologies of the following organs:
- liver;
- kidneys;
- heart.
Transthoracic echography ( ultrasonography) is a non-invasive examination method in which a device sensor is applied to the skin of the chest, and which allows non-invasive determination of pressure in the pulmonary artery system. An increase in pulmonary artery pressure of more than 35 mm Hg indicates pulmonary hypertension and is an indication for right heart catheterization ( a more accurate method of determining pressure, which, however, requires the introduction of a special device into the lumen of the pulmonary trunk).
Determination of pulmonary function
Measurement of vital volume of the lungs ( and a number of other volumes) is an extremely important examination for systemic scleroderma, as it allows one to assess the function of the lungs and the degree of their involvement in the pathology. Pulmonary function tests should be performed every six months to a year.A decrease in the diffusion capacity of the lungs in combination with a decrease in the vital capacity of the lungs indicates restrictive damage to the lung tissue ( decreased elasticity and extensibility of the lungs). An isolated decrease in the diffusion capacity of the lungs indicates damage to the pulmonary vessels due to their sclerosis or due to pulmonary hypertension.
Endoscopy of the gastrointestinal tract
Endoscopy is a diagnostic procedure during which a special flexible endoscope equipped with an optical and lighting system is inserted into the lumen of the esophagus. This examination allows the doctor to assess the condition of the mucous membrane of the esophagus, stomach, and duodenum.With scleroderma, the following changes can be detected:
- reflux of gastric contents into the esophagus due to weakening of the lower esophageal sphincter;
- severe esophagitis ( inflammation of the esophagus);
- fungal infection of the esophagus;
- narrowing of the esophagus;
- Barrett's esophagus ( a precancerous condition that occurs due to irritation of the esophageal mucosa by hydrochloric acid);
- esophageal tumor;
- dilation of blood vessels in the submucosal layer of the stomach.
Capillaroscopy of the nail bed
Capillaroscopy is a method of non-invasively studying the function of capillaries by examining them under a microscope. The fold of the nail bed is usually examined, since in this place the capillaries are close to the surface and are easy to visualize.With scleroderma, fewer capillaries, multiple vascular dilations, and irregular or inverted capillary loops are detected.
Histological examination
Study of fragments of skin and lung tissue ( biopsy) under a microscope allows you to reliably detect sclerotic changes. Histological examination is used when it is necessary to differentiate systemic sclerosis from other similar diseases if other examination methods are not sufficiently informative.With scleroderma, the following changes are detected:
- tissue fibrosis with excessive deposition of collagen and intercellular substance;
- chronic inflammation with infiltration of mononuclear cells ( macrophages and T lymphocytes);
- proliferation of the internal lining of blood vessels, concentric deposits of connective tissue in the wall of blood vessels, narrowing of the lumen of blood vessels, thrombosis.
Treatment of scleroderma with medications
The therapeutic approach for scleroderma depends primarily on the type of disease, the degree of damage to internal organs, as well as the severity of existing clinical manifestations. The current treatment is aimed at eliminating the consequences of sclerotic changes in organs, preventing possible complications, as well as eliminating a number of disturbing symptoms. Unfortunately, a complete cure for this disease is still impossible.
Treatment of scleroderma is carried out using the following groups of drugs:
- enzyme preparations;
- vasodilators;
- drugs that suppress the immune system;
- anti-inflammatory drugs;
- chelation therapy.
Treatment with enzyme preparations
Enzyme preparations are capable of breaking down connective tissue fibers formed in areas of sclerosis. These drugs can be administered systemically in the form of intramuscular injections or locally using electrophoresis. The course of treatment depends on the severity of the disease and can last from 5–7 days to two–three weeks.The following drugs can be used:
- longidase;
- Ronidase;
- lidase;
- trypsin;
- chymotrypsin.
Treatment with vasodilators
The use of vasodilators eliminates spasm of blood vessels, which has a beneficial effect on blood circulation in peripheral tissues and internal organs. This can reduce the severity of Raynaud's phenomenon, pulmonary hypertension, and kidney damage. In addition, vasodilation helps reduce the load on the heart muscle.The following vasodilator drugs are used in the treatment of scleroderma:
- Calcium channel blockers. Calcium channel blockers reduce spasm of vascular smooth muscle by reducing the supply of calcium, a mineral that is necessary for normal muscle fiber contraction. Verapamil tablets are used at a dose of 40–80 mg 3–4 times a day or nifedipine tablets at a dose of 10–20 mg 2 times a day.
- Phosphodiesterase inhibitors. Phosphodiesterase is an enzyme that is involved in the synthesis of a number of substances that can cause vascular contraction. These drugs have the greatest effect on the vessels of the pulmonary circulation, so they are used for pulmonary hypertension. Pentoxifylline is usually used at a dose of 600 mg per day.
Treatment with drugs that suppress the immune system
Since autoimmune processes that arise due to disruption of the body’s immune system play an important role in the development of scleroderma, there is a need to use drugs that suppress the immune system.The following drugs are used to suppress the immune system:
- Cyclophosphamide is a drug that is widely used to treat many autoimmune diseases. Allows you to reduce the intensity of pulmonary fibrosis, slows down the formation of foci of sclerosis in the tissues of internal organs and skin.
- Methotrexate suppresses the division of immune blood cells, due to which it has a pronounced immunomodulatory effect. Research shows its high effectiveness against skin and lung lesions, as well as inflammation of muscles and joints.
- Cyclosporine inhibits the activity of T-lymphocytes, which play a key role in the development of diffuse scleroderma.
- Azathioprine suppresses the metabolism of substances that make up nucleic acids. Thanks to this, it blocks the process of cell division and reduces the intensity of the immune response.
- Rituximab is a drug that specifically blocks B-lymphocytes responsible for the synthesis of antibodies.
It should be borne in mind that the listed drugs have a large number of side effects ( toxic effects on the kidneys, bone marrow, heart, liver, reduce resistance to infections), therefore they should only be prescribed by a competent specialist. Incorrect use of immunosuppressants can cause many serious complications.
Treatment with anti-inflammatory drugs
Treatment with nonsteroidal anti-inflammatory drugs can reduce the inflammatory response and its accompanying symptoms ( swelling, pain, joint damage).Nonsteroidal anti-inflammatory drugs
| A drug | Mechanism of action | Mode of application |
| Diclofenac | Blocks the enzyme cyclooxygenase, which is involved in the breakdown of arachidonic acid at the site of inflammation to biologically active substances that have pro-inflammatory effects. Reducing the level of these substances eliminates the inflammatory reaction, swelling and pain. | Orally 75 - 150 mg in several doses, after meals with plenty of water. |
| Ibuprofen | Orally 800 mg 3 times a day after meals, with plenty of water. | |
| Meloxicam | Orally at a dose of 7.5–15 mg once a day after meals. | |
| Celecoxib | Orally in a daily dose of 200 mg in two doses. |
It should be borne in mind that most non-steroidal anti-inflammatory drugs have a negative effect on the gastric mucosa, and if the dosage regimen is not followed, they can provoke gastritis and ulcers. For this reason, they are often prescribed with stomach-protecting medications ( omeprazole, ranitidine, famotidine).
If non-steroidal anti-inflammatory drugs are ineffective, glucocorticoids - steroid hormones ( dexamethasone, prednisolone), which have great anti-inflammatory activity and can suppress the immune system. The dosage of these drugs is determined strictly individually, since due to the effect on the entire body and due to a number of side effects, their incorrect use can have adverse consequences.
Chelation therapy
Chelation therapy aims to remove certain substances from the body by binding them to specific medications. For scleroderma, D-penicillamine is used, which is able to remove copper from the body. Thanks to this, it is possible to reduce the rate of formation of fibrous tissue and reduce the intensity of the inflammatory reaction ( copper is necessary for the normal functioning of cells that synthesize connective tissue fibers).Hormones and antibiotics for scleroderma
Nutrition for scleroderma ( diet)
Scleroderma is not a disease that requires any specific diet. However, proper nutrition can reduce discomfort and alleviate the general condition of the patient.- if the esophagus is affected, solid foods should be avoided;
- if the intestines are damaged, it is necessary to eat foods rich in plant fibers;
- you should consume a large amount of vitamins and minerals, since their absorption in the intestines is often impaired;
- you need to consume enough calories;
- Large doses of vitamin C should be avoided ( more than 1000 mg/day), as it stimulates the formation of connective tissue;
- in case of kidney damage, it is advisable to reduce the amount of salt and water taken from food.
Is bed rest necessary for scleroderma?
The need for bed rest for scleroderma occurs only when there is severe damage to vital internal organs ( heart, lungs, kidneys), when any physical activity can provoke critical complications. In other cases, there is no need for bed rest, and, moreover, a decrease in physical activity is highly not recommended. This is due to the fact that in the absence of movement, atrophy of the skin and muscles develops faster, and joints lose their mobility.Patients with scleroderma should pay special attention to the temperature regime in which they are located, since cold air can provoke spasm of the blood vessels of the extremities ( Raynaud's phenomenon), which can have many adverse effects.
Traditional methods of treating scleroderma
Traditional methods of treating scleroderma can alleviate the course of the disease, can eliminate some external manifestations, and contribute to a slower development of the disease. However, the use of traditional medicine methods without the use of pharmacological drugs may not be effective enough and lead to various complications.The following traditional methods are used to treat scleroderma:
- Aloe juice. A compress soaked in aloe juice is applied to the pre-steamed skin. The procedure should be repeated once every few days. This product allows you to somewhat reduce the hardness of the skin and the rate of spread of sclerotic lesions.
- Wormwood ointment. Powdered wormwood grass is mixed with fat ( better - with sterile Vaseline) in equal proportions. The resulting ointment must be used to treat scleroderma lesions 2 to 3 times a day for one to two weeks.
- Horsetail infusion. A tablespoon of a mixture of horsetail, knotweed and lungwort should be poured with 200 ml of water and kept in a water bath for a quarter of an hour. After the broth has cooled, it should be strained and drunk 50 ml before meals.
- Decoction of meadowsweet herb. A tablespoon of meadowsweet herb should be poured into two glasses of water and boiled for 7 to 15 minutes. The resulting decoction must be infused for several hours, after which it can be drunk 100 ml twice a day. Reduces pain in joints and areas of inflammation.
- St. John's wort infusion. Three tablespoons of a crushed mixture of St. John's wort, sweet clover, raspberry, plantain and mint should be poured into 5 cups of boiling water and left for 6 to 8 hours, then strain. The resulting infusion should be drunk 50 - 100 ml per day for 2 months.
Answers to frequently asked questions
Can you get infected with scleroderma?
It is impossible to become infected with scleroderma, since this pathology is a consequence of disruption of the normal functioning of the immune system and cells that synthesize connective tissue fibers. Despite the fact that infection can act as a precipitating factor, to date no specific pathogenic agents have been identified that can cause scleroderma.Scleroderma is a chronic inflammatory disease of an autoimmune nature that affects the connective tissue framework of the skin and internal organs. The exact cause of scleroderma is unknown, but it is assumed that this disease is based on a genetic predisposition that is activated under the influence of certain external or internal factors.
The genetic predisposition is based on a defect in the genes responsible for the formation of structures necessary for the recognition of cells and tissues in the self-foe system during the immune response. As a result of changes in the structure of genes, a protein is formed that is unknown to the human immune system, which leads to the fact that immune cells begin to attack their own tissues. This leads to the development of inflammation and to the excessive release of factors that stimulate the formation of connective tissue.
Changes in the structure of the genetic apparatus can occur under the influence of the following factors:
- ionizing radiation;
- ultraviolet radiation;
- nitrates;
- nutritional supplements;
- viruses;
- some medications.
As evidence of the genetic nature of scleroderma, there is a high incidence of this disease among close relatives and identical twins. However, numerous studies indicate that in most cases the genetic defect manifested itself only after exposure to some external or internal provoking factor.
The development of scleroderma can be triggered by the following factors:
- Inflammation. The inflammatory response is a factor that, in the presence of a genetic predisposition, can cause the development of systemic scleroderma. This is explained by the production of pro-inflammatory substances that can stimulate fibroblasts - cells that synthesize connective tissue fibers. Against the background of excessive activity of these cells, “dormant” genes are activated, which begin to synthesize the defective protein. As a result, an immune response to one’s own tissues is triggered, which stimulates the further development of the disease.
- Autoimmune process. An autoimmune process is a pathological condition in which a person’s immune system attacks his own tissues.
- Infection. Some infectious agents have an antigenic structure similar to normal tissues of the human body. This means that under certain conditions an autoimmune reaction can occur. However, it should be understood that such a pathological response in scleroderma is possible only in the presence of a genetic predisposition.
- Environment. Some environmental factors, such as salts of heavy metals, silicon, organic solvents, benzene and toluene, in the presence of predisposing factors, can cause the formation of foci of sclerosis in the skin and internal organs.
- Medications. Some medications ( bleomycin, cocaine, diltiazem, etc.) can cause activation of defective genes, which can lead to the development of systemic scleroderma.
What do people with scleroderma look like?
The appearance of patients with scleroderma depends on the location of sclerotic lesions, the degree of damage to the skin and limbs, the condition of subcutaneous fat, muscles and bones, as well as the stage of development of the disease.In the development of scleroderma, one should distinguish between the initial stage, during which the inflammatory process is active, and a later stage, during which the intensity of inflammation decreases and sclerotic and atrophic foci develop.
Scleroderma can affect the skin, blood vessels and some internal organs. Depending on the appearance and location of sclerosis foci on the skin, several types of this disease are distinguished.
Types of scleroderma
| Form of scleroderma | Description | Photo |
| Plaque scleroderma | Initially, swollen pinkish round plaques up to 5 cm in size appear on the skin, which gradually increase in size. A blue-violet border zone is observed on the periphery of these outbreaks. As the lesions grow, the skin in the center thickens and acquires a yellowish tint. |  |
| Linear scleroderma | The linear lesion is located on the scalp. Apply to facial skin - forehead, nose, cheeks. The skin in the area of the lesion is thin and pale. |  |
| White spot disease | Small shiny lesions up to one centimeter in size. They are located on the body and mucous membranes of the oral cavity. |  |
| Bullous scleroderma | Bubbles with transparent contents, which are combined with other forms of sclerotic foci. |  |
| Limited scleroderma with unilateral facial atrophy | The sclerotic lesion covers the skin around the eyes, in the area of the zygomatic arch and lower jaw. Due to atrophy of the underlying muscles and bones, facial asymmetry occurs with a decrease in the affected side. |  |
In addition to the listed specific formations on the skin, with scleroderma the limbs may suffer with the formation of ulcers, atrophy of muscles and bones. Often, foci of sclerosis are found in the neck, face, torso, and genitals.
Damage to the limbs and trunk with scleroderma
| Type of lesion | Description | Photo |
| Ulcers on fingers | Sores form on the pads of the nail phalanges of the fingers ( open defects) of various sizes and shapes. When an infection occurs, suppuration may occur. |  |
| Hand muscle atrophy | Due to atrophy of the muscles and subcutaneous fatty tissue, bones are easily visible on the surface of the hand; the fingers become like “bamboo branches” due to the visual highlighting of the surface of the joints. Sometimes thickening of the bones of the nail phalanges occurs ( drumstick-shaped fingers). |  |
| Muscle atrophy of the lower limb | Atrophy of the muscles and subcutaneous tissue below the knee level occurs, making the lower leg look thinner. In some cases, the muscles atrophy so much that the skin is directly adjacent to the bone. |  |
| Thickening of the neck skin | The skin in the neck area is thickened and inactive, has a yellowish tint. Due to decreased elasticity, the skin cannot be folded. The surface of the skin is dry and cold. |  |
| Thickening of the skin around the mouth | The skin around the mouth is thickened and its elasticity is reduced, which is why the opening of the mouth is limited. |  |
With scleroderma, disorders are not limited to the skin. Damage to the blood vessels develops, which is manifested by spider veins and Raynaud's phenomenon.
Vascular damage in scleroderma
| Type of vascular lesion | Process stages | Description | Photo |
| Raynaud's phenomenon | Paleness of fingers | The fingers are pale, sharply different in color from the hand. |  |
| Blueness of fingers | Fingers become bluish. |  |
|
| Redness of fingers | Fingers are red. With scleroderma, this stage may be absent. |  |
|
| Spider veins | – | Small reddish branched dots formed by superficial capillaries. Found on the skin of the chest, shoulders, mucous membranes of the mouth, and genitals. |  |
There is a curious and witty aphorism, said to have been coined by French doctors, which states that a person lives as many years as his blood vessels live. Needless to say, it is said expressively and quite aptly. Indeed, from the time when a network of blood vessels in any part of the body fails, its vital activity deteriorates sharply or even the death of cells in this area occurs if collateral vessels, i.e., peculiar vessel-channels, do not have time to pump blood here from other highways and will not provide nutrition to tissues. This is the essence of myocardial, brain, kidney, lung, etc. infarctions.
The capillary network is one of the most important components of the human cardiovascular system and the body as a whole. It includes more than 100 billion capillaries with a total length of more than 100,000 km with an exchange surface area of more than 6,000 square meters. m. It is in the capillary network that the exchange of oxygen and nutrients between vessels and cells takes place - tissue metabolism. Capillary blood flow is called microcirculation.
The course of almost any disease is accompanied by certain changes in the microcirculation system, capillary morphology, capillary blood flow speed, which, as a rule, entails metabolic disorders of the corresponding organs and tissues.
According to modern medical research, it is generally accepted that disruption of the microcirculatory system leads to premature aging and is the cause of many diseases. More than 80% of all human diseases are due to poor blood flow or its absence in the peripheral circulatory system.
Capillaroscopy- practically the only opportunity to accurately determine the causes of symptoms that signal very serious diseases. But millions of people are accustomed to enduring them rather than treating them:
- deterioration of health,
- headache,
- fainting,
- disruption of the cardiovascular system,
- hand diseases,
- rachiocampsis.
Compared to other diagnostic methods, capillaroscopy has the advantage of preventing development rather than eliminating the consequences:
- ischemic changes in the heart and brain,
- diabetes mellitus
- hypertension
- kidney pathologies.
Using capillaroscopy it is easy to:
- anticipate the development of diseases,
- evaluate the effectiveness of treatment,
- record changes that occurred after a course of procedures or medications.
Capillaroscope(from capillary and Greek - I look) - a testing device, a type of microscope for monitoring the condition of the smallest vessels in the body - capillaries. The capillaroscope is used for non-invasive examination of human capillaries. Through the lens of a capillaroscope, a specialist evaluates the density of capillaries, their shape, location, and thickness in diameter. Deviations from the ideal shape, uneven blood filling, clots, and a large number of shadow capillaries indicate the presence of pathology. For example, if there are more than 20% of shadow vessels, there is a risk of heart attack, stroke and even coma.
Capillaroscopy is a unique research method:
- highly accurate – diagnostics eliminates misdiagnosis;
- bloodless and painless - the study is carried out through the skin;
- safe - there is no risk of infection, radiation, there are no contraindications;
- fast – the examination takes 5 minutes;
- unique - other devices are unable to “see” the capillaries;
- capillary blood flow is observed in the “natural environment”, which increases the accuracy of diagnosis.
Capillaroscopy, unlike other morphological research methods (X-ray, ultrasound), gives an idea of the state of the whole organism, reflects metabolic disorders, allows us to predict the development of the disease at preclinical stages, finely quantify changes in the condition over time, and select therapy.
Full visualization and measurement of capillary parameters and capillary blood flow is possible only with the help of capillaroscopy.
“I see signs of vegetative-vascular dystonia, slight swelling in the tissues and slightly increased blood density,” explains the therapist, examining my ring fingers under a microscope. You don’t need serious treatment, but your capillaries “ask” to slightly adjust your lifestyle: move more, get proper rest and increase fluid intake.” We introduce you to a new type of analysis, which some call capillaroscopy of the nail bed - then you will find out what can be learned from the diagnosis and where to do capillaroscopy. It’s hard to argue: there are still not enough walks in the fresh air in the metropolis, in the “work-home” mode. And, the doctor says, you need to walk for at least two hours a day, and the journey to work and back is not taken into account. She explains: weakened blood circulation, which results from physical inactivity, depletes the nutrition of tissues and organs, forming edema. It is also worth taking care of proper sleep and consuming at least two liters of water during the day.
“You don’t need to be afraid that you drink a lot of water, and in the morning it will “gather” under your eyes,” says the doctor. – If there are no complaints about the functioning of the kidneys, the liquid will come out without problems in the urine and then. Salt retains water in the body - remember this when planning your diet. Avoid excesses in salty dishes." I learned this truth about myself after several minutes of examination “under a microscope.” Before pointing the magnifying glass, the doctor lubricates the cuticles on the ring fingers with camphor oil. And within a few minutes the capillaries tell the specialist about all the hidden problems in the “experimental” body. Capillaroscopy allows you to safely (there is no risk of infection), informative, accessible and understandable even for the patient to assess the condition of the vessels of the whole body, to identify possible malfunctions in the functioning of internal organs, explains the doctor. With its help, we determine how the blood supply to the brain occurs, how the pumping function of the heart operates, the pressure, speed and density of blood, and whether there is swelling in the tissues. The method also makes it possible to determine congenital vascular anomalies in newborns and to assess the level of endurance of an adult’s body. Nail bed capillaroscopy examines the vein capillaries. “I often repeat to both colleagues and patients that capillaries are a kind of arbiter of the well-being of the cardiovascular system. Human health is determined by the condition of the body’s small vessels. If everything is in order with the capillaries, then the cardiovascular system is normal. Their condition reflects the biological age of the individual. The lack of blood supply to the capillaries (the so-called shadow capillaries) indicates early aging of the body. If the number of shadow capillaries exceeds 30%, the patient has a high risk of developing vascular critical conditions of a catastrophic nature - stroke, heart attack, which is equivalent to a car accident when the car cannot be repaired or restored.” The doctor claims: absolutely all diseases of the cardiovascular system begin with disorders in the capillaries. A capillaroscope, a highly sensitive device made using the latest technologies, helps to notice changes in the capillary network. With its help, vascular diseases are diagnosed at the earliest stages, when the clinical picture is still poorly expressed. And if the problem is recognized at this level, the person can be treated quickly and effectively; these are the main tasks of capillaroscopy of the nail bed. “Of the 200 patients who underwent capillaroscopy, every fifth had severe signs of blood supply deficiency, every seventh had atypical forms of capillaries, every third suffered from impaired arteriolar tone, severe spasm, every second had certain signs of venous stagnation of varying degrees,” says she. This is an alarming beacon that indicates serious problems with the health of citizens.” It is important to identify improper blood circulation in the capillaries and veins. It turns out that the human body has a hundred thousand kilometers of capillaries! Each capillary is like a microheart. 10000000000 micro hearts work daily, delivering nutrients to tissues and removing harmful metabolic products. Only small vessels can do this. If blood circulation in the capillaries and veins is disrupted, blood stagnation and metabolic failure will occur. Therefore, immunity will decrease, chronic diseases will worsen and new diseases will arise, sleep and concentration problems will occur, and memory loss will occur. “Even short-term loss of consciousness, arrhythmias, coldness of the fingers, instability of blood pressure and pulse are possible,” she warns. “If blood circulation in the capillaries stops, tissue will begin to die.” The brain, spinal cord and skeletal system are the most sensitive to circulatory disorders, so the patient’s first sensations are headaches, pain in the bones and joints.
If, after treatment or a change in lifestyle, the capillaries are restored, the person feels rejuvenated, the impression that they have “shed” a dozen years. This is noticeable even visually: your posture changes, memory improves, and endurance increases. If the grandmother previously only sat and watched her grandchildren during a walk, then after the “correction of the capillaries” she already accompanies the baby. A person’s mood improves and depressive states disappear as if by hand. Of course, if you constantly monitor the condition of your body and treat it on time (if necessary), then there is no need to worry. “In order to have healthy capillaries, it is necessary to regularly (and not from time to time) engage in physical activity, sports, not overeat, do not abuse alcohol and avoid low-quality products,” the specialist notes. Try to rationally combine working time and active rest, and have adequate sleep (8-10 hours a day). Balances possible stress with joyful moments in life - communicate more with people you like, read interesting books, travel, spend time on your favorite hobbies.” Capillaroscopy is the filming of capillaries visible on the fingers or on the surface of the eye with a special device. Its advantage is that the doctor observes capillary blood flow in a “natural environment,” and this increases the accuracy of diagnosis. The procedure is painless, has no contraindications and takes only 15 minutes. A healthy capillary is ideally like a “lady’s stiletto heel”